Genes associated with “FXTAS

52 genes foundOpen Targets: fragile X-associated tremor/ataxia syndrome
How are genes scored? (0–100 composite)
-5–15
ClinGen
0–15
HPO Freq
0–15
Open Targets
0–12
Phen2Gene
0–10
ClinVar
0–8
Constraint
0–8
Dosage
0–8
OMIM CS
0–8
PanelApp
0–5
OMIM
0–5
G2P
0–4
Breadth
Tiers:Strong Candidates (≥20)Consider (≥8)Possible (≥3)
Evidence dots:
HPOClinVarPhen2GeneOpen TargetsPanelApp
hover for details

Strong Candidates

1 gene
1
FMR1

fragile X messenger ribonucleoprotein 1

29
score
ClinGen: DefinitiveGTR ↑

Fragile X syndrome

Frequency
-
P/LP Variants
18
OT Score
0.67

Consider

9 genes

FRAGILE X TREMOR/ATAXIA SYNDROME; FXTAS

12SCN8A
Def

sodium voltage-gated channel alpha subunit 8

10PMP22
Def

peripheral myelin protein 22

9PURA
Def

Neurodevelopmental disorder with neonatal respiratory insufficiency, hypotonia, and feeding difficulties

8GABRA1
Def

gamma-aminobutyric acid type A receptor subunit alpha1

8GABRB3
Def

gamma-aminobutyric acid type A receptor subunit beta3

8SPTBN4
Def

spectrin beta, non-erythrocytic 4

8ATP2B2
Def

ATPase plasma membrane Ca2+ transporting 2

?Inclusion body myopathy with early-onset Paget disease with or without frontotemporal dementia 2

Possible

23 genes — click to expand

Neuronal intranuclear inclusion disease

glutamate metabotropic receptor 1

6MECP2
Def

methyl-CpG binding protein 2

CUGBP- AND ELAV-LIKE FAMILY, MEMBER 1; CELF1

gamma-aminobutyric acid type A receptor subunit alpha5

ataxin 7

4GABRG2
Def

gamma-aminobutyric acid type A receptor subunit gamma2

4CSTB
Def

cystatin B

4RELN
Def

reelin

4ASPA
Def

aspartoacylase

4GABRB2
Def

gamma-aminobutyric acid type A receptor subunit beta2

4PSAP
Def

prosaposin

4DST
Def

dystonin

4KCNA1
Def

potassium voltage-gated channel subfamily A member 1

ATP/GTP binding carboxypeptidase 1

4GALC
Def

galactosylceramidase

calcium voltage-gated channel subunit alpha1 A

3GABRD
Mod

gamma-aminobutyric acid type A receptor subunit delta

3ZNF423
Mod

zinc finger protein 423

calcium voltage-gated channel auxiliary subunit alpha2delta 2

Data aggregated from HPO/Monarch, ClinVar, OMIM (phenotypes + clinical synopsis), Open Targets, PanelApp, Phen2Gene, and Gene2Phenotype. Click a gene to view full details.